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PEDIATRIC BRS > heme/onc > Flashcards

heme/onc Flashcards

1
Q

low reticulocyte count indicates what?

A

bone marrow failure or diminished hematopoiesis

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2
Q

clinical features of iron deficiency anemia in adolescents

A

spoon shaped nails and diminished attention and ability to learn

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3
Q

low serum ferritin results in INCREASED/DECREASED transferrin?

A

increased transferrin and decreased transferrin saturation

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4
Q

what should iron be ingested with?

A

vitamin C to enhance intestinal iron absorption

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5
Q

anemia characterized by defective synthesis of one of the Hgb Chains?

A

thalassemia

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6
Q

type of thalasemia prevalent in south east asians?

A

alpha thalassemia

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7
Q

two alpha glob in genes are deleted and have mild anemia

A

a thalassemia minor

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8
Q

three alpha glob in genes are deleted - patients have severe anemia at birth .

A

Hemoglobin H disease

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9
Q

what is hemoglobin Barts ?

A

seen in three alpha glob in genes deletion

binds oxygen very strongly doesn’t release it to tissue

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10
Q

four alpha glob in genes deleted resulting in only hgb harts formation

A

fetal hydrops

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11
Q

hepatosplenomegaly
bone marrow hyperplasia
maxillary hyperplasia w/ prominent cheekbones and skull deformities

A

b thalassemia major

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12
Q

what population has b thalassemia major?

A

mediterranean background

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13
Q

what is the treatment of beta thalassemia major?

A

lifelong transfusions and splenectomy

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14
Q

what is a complication of beta thalassemia major? what drug can help it?

A

hemochromatosis

deferoxamine

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15
Q

Beta thallasemia minor is commonly misdiagnosed as what?

A

iron deficiency anemia

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16
Q

whmild asymptomatatic anemia w/ hemoglobin level 2-3 below age appropriate norms and target cells and anisocytosis?

A

beta thalassemia minor

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17
Q

characterized by prussian blue staining resulting from accumulation of iron in mitochondria of RBC precursors in bone marrow ?

A

sideroblastic anemia

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18
Q

macrocytic megaloblastic anemias are characterized with MCV > ?

A

95

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19
Q

what are two major causes of macrocytic megaloblastic anemia?

A

folic acid and vitamin B 12 deficiency

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20
Q

vitamin b 12 must first combine with what?

A

instrinsic factor secreted by gastric parietal cells

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21
Q

where is vitamin b 12 absorbed?

A

terminal ileum

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22
Q

what is up w/ tongues in vitamin b 12 deficiency?

A

smooth red tongue and neurology manifestations

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23
Q

what is the treatment of vitamin b 12 def?

A

monthly IM vitamin b 12 injections

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24
Q

spectrin defect causing RBC membrane instability autosomal dominant?

A

hereditary spherocytosis

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25
Q

splenomegaly, pigmentary gallstones, aplastic crises and high retic count ?

A

hereditary spherocytosis

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26
Q

what studies are used for hereditary spherocytosis ?

A

osmotic fragility studies

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27
Q

tx of hereditary spherocytosis

A

transfusions and splenectomy after 5 years of age

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28
Q

what are 2 enzyme deficiencies of RBS?

A

pyruvate kinase and G6PD

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29
Q

what does blood smear show in G6PD deficiency?

A

bite cells and hemighosts/ heinz bodies

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30
Q

what are some triggers of hemolysis in G6PD

A

fava beans, infection, drugs

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31
Q

positive direect coombs test indicates?

A

autoimuno hemolytic anemia

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32
Q

tx for autoimmune hemolytic anemia

A

corticosteroids and transufision

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33
Q

occurs when mom is Rh negative has Rh positive baby and forms Rh antibodies and subsequent pregnancies result in hemolysis of Rh positive babies? with a strongly positive coombs test

A

Rh hemolytic disease

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34
Q

this type of alloimmune hemolytic anemia can occur in the first pregnancy when mom is blood group O and fetus is blood group A, B, or AB? Moms antibodies are passed downy o baby causing hemolysis ?

A

ABO hemolytic disease

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35
Q

what is management for alloimmune hemolytic anemia?

A

phototherapy and exchange transfusion

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36
Q

cause SS

A

single amino acid substitution of valine for glutamic acid on number 6 position of B glob in chain of Hgb

37
Q

what leads to distorted RBC shape in SS?

A

polymerization of HgB

38
Q

2 genes for Hgb S?

A

SS disease

39
Q

SS trait

A

1 gene for Hgb S

40
Q

what is the hemoglobin makeup for those w/ SS trait?

A

Hgb A, Hg S and some HgF

41
Q

diagnosis of SS disease?

A

Hg electrophoresis

42
Q

what should you always suspect in a person presenting w/ priapism?

A

sickle cell

43
Q

parvovirus B 19 can cause?

A

aplastic crisis ( SLAPPED CHEEKS)

44
Q

leading cause of death from SS disease?

A

infection (sepsis or meningitis)

45
Q

red blood life span SS

A

10-50 days

46
Q

hemoglobin in SS

47
Q

hematocrit in SS

48
Q

reticulocyte count in SS

49
Q

abc count in SS

A

12,000-20,000

50
Q

platelet count in SS

51
Q

bili in ss

52
Q

blood smear in SS

A

sickled cells, target cells, howell jolly bodies

53
Q

bone marrow in SS

A

erythroid hyperplasia

54
Q

treatment in SS?

A

hydroxyurea, oral penicillin, daily folic acid, routine immunizations, serial transcranial doppler ultrasound

55
Q

other name for fanconi anemia?

A

congenital aplastic anemia

56
Q

inheritance for fanconi anemia

A

autosomal recessive

57
Q

what are the skeletal abnormalities of falcon anemia?

A

absence or hypoplasia of the thumb and radius

58
Q

what are lab findings in pancytopenia?

A

decreased RBCs, leukocytes and platelets

59
Q

sulfonamides, anticonvulstants, chloramphenical, HIV, EBV and CMV chemicals and radiation may cause ?

A

acquired aplastic anemia

60
Q

increase in RBC’s relative to total blood volume

A

polycythemia

61
Q

apparent increase in RBC mass caused by a decrease in plasma volume

A

relative polycythemia

62
Q

what is the most common cause of relative polycythemia

A

dehydration

63
Q

prolonged PTT and normal PT w/ hemarthroses?

A

hemophilia A ( factor 8 deficiency)

64
Q

management of hemophilia A

65
Q

no hemarthrosis, mucosal bleeding, menorrhagia

A

vvon wile brand disease

66
Q

diagnosis of vwf ?

A

ristocetin cofactor assay

67
Q

management of vwf?

68
Q

factor 9 deficiency

A

hemophilia B

69
Q

what is vitamin K responsible for?

A

factors II, VII, IX and X proteins C and S

70
Q

what is PTT and PT in vitamin K deficiency?

A

prolonged in BOTH

71
Q

tx for vitamin K def?

A

IM vitamin K prevents hemorrhagic disease

72
Q

accelerated fibrinogenesis and fibrinolysis

73
Q

thromboycytopenia, prolongation of PT and PTT, reduction in clotting factors and elevated fibrin (D - DIMER)

74
Q

thrombocytopenia, unusually small platelets, excezma and defects in T and B cell immunity

A

wiscott aldrich syndrome

75
Q

how can you distinguish between tar and fanconi?

A

TAR has a thumb !

76
Q

what is the treatment for ITP?

77
Q

what causes ITP?

A

viral infection

78
Q

what is the treatment for chronic ITP

A

splenectomy

79
Q

when mom has thrombocytopenia

A

neonatal immune mediated thrombocytopenia

80
Q

when mom doesn’t have thrombocytopenia but still passes antibodies against fetuses platelets

A

isoimmune thrombocytopenia

81
Q

kasabach merrit syndrome

A

when large hemangiomas sequester and destroy platelets

82
Q

diminished ability of platelets to aggregate and forma clot bc of deficient adhesive glycoprotein IIB/IIIa

A

glanzmann’s thrombasthenia

83
Q

absent platelet membrane glycoproteins and vwf?

A

bernard soulier syndrome

84
Q

noncyclic neutropenia with mild infections w/ low ANC

A

chronic benign neutropenia of childhood

85
Q
A

kostmann syndrome ( severe congenital agranulocytosis

86
Q

recurrent fever, oral ulcers, stomatitis diagnosis?

A

cyclical neutropenia diagnosed by serial neutrophil counts

87
Q

short stature, immunodeficiency fine hair and neuropenia

A

cartilage hair hypoplasia syndrome

88
Q

exocrine pancreatic insufficiency w/ short stature caused by metaphyseal chondrodysplasia and neutro penia w/ constant otitis media?

A

schwachman diamond syndrome

PEDIATRIC BRS (9 decks)

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